Lung function and aerobic capacity in adult patients following modified Fontan procedure

Objective-To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation. Design-Retrospective cohort study. Values of maximum oxygen uptake (Vo(2)ma x), maximum heart rate (HRmax), forced vital capacity (FVC), and forced exp iratory volume in one second (FEV1) were compared with predictive values fo r different age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle cond uit (RA-RV). Results-At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished Vo(2)max, HRmax, FVC, and FE V1 compared with predicted values. Early age at surgery had a positive impa ct on aerobic capacity. The FEV1:FVC ratio indicated restrictive lung funct ion. No differences were found with respect to any variable between patient s with RA-PA connections and those with RA-RW connections. Conclusions-Patients with a Fontan circulation have greatly diminished valu es of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of Vo(2)max. The t heoretical benefits of including the right ventricle in a Fontan circulatio n were trot apparent.